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Propriospinal Myoclonus

  • Roongroj Bhidayasiri
  • Daniel Tarsy
Chapter
Part of the Current Clinical Neurology book series (CCNEU)

Abstract

Propriospinal myoclonus (PSM) is a rare movement disorder characterized by myoclonic jerks in muscles believed to originate in a myoclonic generator (a “myelomere”) which spread rostrally and caudally to other myotomes above and below the generator. Characteristic features include repetitive, arrhythmic flexion jerks of the trunk, hips, and knees. The signal spreads slowly along propriospinal pathways. Propriospinal myoclonus is idiopathic and has occurred following spinal cord lesions, spinal trauma, drug use, tumor, or infections. However, a specific cause is identified in only 20% of patients. Most affected patients are middle-aged men. In most cases, the myoclonic generator is at the thoracic level. Diagnosis is based on the characteristic clinical features mentioned above and polymyography which shows a slow and orderly rostrocaudal propagation of muscle recruitment.

Keywords

Affected Patient Spinal Cord Lesion Spinal Trauma Symptomatic Therapy Myoclonic Jerk 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Supplementary material

The video demonstrates that cutaneous stimuli to the back, chest, and abdomen elicit axial myoclonic jerks which cause low-amplitude flexion movements of the hips, neck, trunk, and right sternomastoid muscle while the patient is standing. There is mild scoliosis.

Propriospinal myoclonus.mp4 (MP4 12,197KB)

References

  1. 1.
    Brown P, Thompson PD, Rothwell JC. Axial myoclonus of propriospinal origin. Brain. 1991;114:197–214.PubMedGoogle Scholar
  2. 2.
    Roze E, Bounolleau P, Ducreux D, et al. Propriospinal myoclonus revisited. Clinical, neurophysiologic, and neuroradiologic findings. Neurology. 2009;72:1301–9.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  • Roongroj Bhidayasiri
    • 1
    • 2
  • Daniel Tarsy
    • 3
  1. 1.Chulalongkorn Center of Excellence on Parkinson’s Disease and Related DisordersChulalongkorn University HospitalBangkokThailand
  2. 2.Department of NeurologyDavid Geffen School of Medicine at UCLALos AngelesUSA
  3. 3.Department of NeurologyHarvard Medical School Beth Israel Deaconess Medical CenterBostonUSA

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